Detection of 14-3-3 protein in the CSF of a patient with Hashimoto’s encephalopathy

Abstract

The mean (ϮSD) MMSE score at Visit 1 was 18.1 Ϯ 3.4 and at Visit 2 was 18.3 Ϯ 3.4. There was a very small but significant improvement (0.2 Ϯ 2.1 points, p Ͻ 0.0001) in MMSE score from first to second test. The figure illustrates distribution of the difference in MMSE scores between Visits 2 and 1. The most commonly observed (mode) change was zero and was observed in 24% of subjects. Variations Ն3 points were seen in 22% of subjects. Variations Ͼ4 points were seen in 4.1% of subjects; therefore, approximately 95% of the subjects in our sample showed a short-term change of Յ4 points on the MMSE.There is a small short-term test-retest variability in the MMSE score in AD that likely reflects a learning effect, cognitive fluctuations, measurement error, and other factors. In addition to age, gender, and educational level, such variability must be considered while screening for dementia or monitoring changes or performing both over time.

The neurological disorder associated with thyroid autoimmunity

“…Dementia, depression, cerebellar symptoms, pyramidal symptoms and extrapyramidal symptoms are typical of the progressive variety of HE. The cognitive decline differs according to the age of patients [2,8,10,18,19,21,22,23,26,27,30,31,33,34,37,38,39,44,45,49,55,57,60,61,66,68,70,72,73,77,84,88]: children are described as badly performing at school, showing learning difficulties or poor concentration [49,84,88], whereas adult patients can suffer a memory impairment as well as progressive aphasia or apraxia. In one case, the severity of symptoms was such that the patient was given a diagnosis of Alzheimer's disease [33].…”

“…High-amplitude theta-delta rhythms can be superimposed over the slow background with a symmetrical or focal distribution. Epileptiform discharges are rarely described in HE, in spite of the high frequency of seizures [1,21,37]. Focal spikes were reported in a confused and paranoid patient who had no seizures [17].…”

“…PET scans revealed global hypometabolism in one patient with cognitive decline and confusion [70]. The remaining patients showed focal areas of hypoperfusion or metabolism abnormalities that had not a clinical correlate [7,18,36,37,42,43,62,79]. SPECT and PET findings did not correlate with the EEG or neuroradiologic findings and were not interchangeable: in one patient who developed confusion, tremor, myocloni and stupor, SPECT was normal while PET showed biparietal hypometabolism [36].…”

Immunoglobulins in Urine of Hamsters with Scrapie

“…Moreover, it seems doubtful that measuring protease-resistant IgG bands in urine will form the basis for a useful diagnostic test for prion disease. Such measurements will be fraught with all the difficulties encountered by medical investigators who have attempted to use elevated levels of the stress protein 14 -3-3 in cerebrospinal fluid as a diagnostic marker for prion disease (5,26,27).…”

Imaging of prion diseases

“…This CSF marker appears more sensitive than EEG with a reported sensitivity of approximately 85% (34,35). However the specificity is relatively low, with multiple false positive results including Hashimoto encephalopathy (36), paraneoplastic encephalopathy (37), herpes encephalitis, cerebrovascular disorders (38), Alzheimer's disease and lymphoma (34) among others. Given the limited accuracy of each tests, a multimodal approach is favored, including the parallel use of EEG, CSF protein 14-3-3 and MRI (31,39).…”